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Sickle Cell Anemia/Hemoglobin SS Disease
Published in Charles Theisler, Adjuvant Medical Care, 2023
Early signs and symptoms of sickle cell disease include swelling of the hands and feet; symptoms of anemia, including fatigue, or extreme tiredness; and jaundice. Over time, sickle cell disease can lead to complications such as infections, delayed growth, and episodes of pain, called pain crises.1 Neurologic complications are also common in sickle cell disease and include ischemic and hemorrhagic stroke, seizures, CNS infection, hearing loss, cognitive impairment, and, rarely, spinal cord infarction.2 SCD can cause lifelong disabilities and reduces average life expectancy to about 40 years in the U.S.
Sickle Cell Disease
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
Sickle cell disease is an autosomal recessive disease resulting from an alteration in the structure of hemoglobin producing hemoglobin S (HbS). It is characterized by chronic hemolytic anemia and vaso-occlusive events.
Anemia: Approach to Diagnosis
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Sickle cell and other hemoglobinopathies result in extravascular hemolysis and accompanying elevations of serum lactate dehydrogenase and total and unconjugated bilirubin. Hemoglobin crystals and target cells in hemoglobin C disease and the sickle cells of sickle cell SS disease are typical abnormalities of anisocytosis. Heterozygous sickle cell disease may be identified by a sickle cell prep, and hemoglobin electrophoresis will help to identify other abnormal hemoglobins. Anemias combining the disorders of sickle cell disease, iron deficiency, thalassemia, hemoglobin C disease, and red cell enzymophathies present challenging diagnostic problems requiring additional laboratory testing including free erythrocyte protoporphyrin, transferrin saturation, hemoglobin electrophoresis, enzymatic, and genetic studies. When other causes of hemolysis are excluded and there is no evidence of abnormal hemoglobin, a red cell enzymopathy should be considered. Congenital or acquired defects may be found, as in patients with pyruvate-kinase deficiency, which may be associated with the development of preleukemia.
Attitudes and practices of unmarried adults towards sickle cell disease: emergent factors from a cross sectional study in Nigeria’s capital
Published in Hematology, 2022
Obi Peter Adigwe, Godspower Onavbavba, Solomon Oloche Onoja
Globally, the prevalence of the disease is highest in sub-Saharan Africa, with Nigeria saddled with the highest burden of carrier prevalence ranging from 25% to 30% [5,6]. Furthermore, in the country, sickle cell anaemia occurs in approximately 3% of all births [7–9]. Some of the common characteristics of sickle cell disease include chronic haemolytic anaemia and recurrent vaso-occlusion, with the latter being responsible for painful crises associated with the disease. Another common feature of the condition is chronic vasculopathy triggered by free heme resulting in nitric oxide scavenging and upregulation of adhesion molecules in reticulocytes neutrophils and endothelial cells [10,11]. One of the major causes of mortality among children is overwhelming bacterial infections occurring due to encapsulated organisms, particularly pneumococcus [1213]. Other common causes of death include splenic sequestration [14], acute chest syndrome [15], stroke [16,17] and multiple organ failure [18]. There is therefore substantial evidence that the disease constitutes a high mortality risk for under-five sickle cell disease patients who live in areas without adequate access to healthcare [19]. This is further complicated by the fact that empirical evidence now correlates malaria episodes with increased risk of crises, alongside the possible morbidity and mortality associated with these events [20,21].
Audit of clinical and laboratory parameters of hemoglobin SS patients in a Nigerian teaching hospital
Published in Annals of Medicine, 2022
Titilola Stella Akingbola, Oladapo Wale Aworanti, Sunday Peter Ogundeji
As the survival improves, sickle cell disease patients are predisposed to different complications of the disease such as chronic leg ulcer, priapism, chronic osteomyelitis, acute chest syndrome, stroke, pentazocine addiction. In these participants, the commonest complication reported was chronic leg ulcer, this is seen in about 12% of the total participants. The cause of leg ulcer is the vaso-occlusion of skin microvasculature, worsened by trauma, infection, warm climate, and iron overload. This is consistent with the findings of Bazuaye et al. where the incidence of chronic leg ulcer was about 10% [26]. Next to the chronic leg ulcer is priapism, seen in about a quarter of male respondents. Previous findings showed prevalence of priapism to be about 45% in SCD male patients [27], our finding in this study is relatively lower compared to the findings by Nwogoh et al. [28]. The least complications seen were pentazocine addiction, sickle cell nephropathy and infertility.
Role of Oxidative Stress and the Protective Effect of Fermented Papaya Preparation in Sickle Cell Disease
Published in Hemoglobin, 2022
Prashant P. Warang, Nikhil S. Shinde, Vinod D. Umare, Prajyot V. Deshmukh, Kanjaksha Ghosh, Manisha R. Madkaikar, Roshan B. Colah, Malay B. Mukherjee
Hematological indices were measured on an automated blood cell counter (K-1000; Sysmex Corporation, Kobe, Japan). Hb analysis was done using high-performance liquid chromatography (HPLC) on the VARIANT™ Hemoglobin Testing System (Bio-Rad Laboratories, Hercules, CA). The ROS assay using 2,7-dichlorofluoresin diacetate (DCF) fluorescent dye test, LP assay using DHPE [N-(fluiorescein-5-thiocarbomaoyl)-1,2-dihexadecanoyl-sn-glycero-3-phosphoethanolamin, triethylammonium salt] dye and PS externalization using fluorescein-labeled Annexin V, were performed by flow-cytometry analysis [3,4], while GSH levels were measured by spectrophotometry. Red cell membrane protein abnormalities were studied by flow-cytometry using eosin 5′ malaemide (E5′M) dye. Red cell morphological changes due to oxidative stress and after FPP treatment were also seen by scanning electron microscopy (SEM). Untargeted metabolites of FPP were measured using liquid chromatography-mass spectrometry (LCMS). FPP was obtained from Osato Research Institute, Gifu, Japan. Oxidative stress was generated in vitro using 2,2′-Azobis (2-methylpropionamidine) dihydrochloride (AAPH). Statistical analysis was performed using GraphPad Prism version 4 software (https://www.graphpad.com/scientific-software/prism/) and Microsoft Office Excel. The hematological and biochemical data of the sickle cell disease patients and healthy individuals are summarized in Table 1. Sickle cell disease patients had low level of Hb, whereas white blood cell (WBC) counts, reticulocyte counts, and total bilirubin levels were increased when compared to healthy individuals.